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MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation
König, Nicole, Stetefeld, Henning R., Dohmen, Christian, Mergenthaler, Philipp, Kohler, Siegfried, Schönenberger, Silvia, Bösel, Julian, Lee, De-Hyung, Gerner, Stefan T.
, Huttner, Hagen B., Schneider, Hauke
, Reichmann, Heinz, Fuhrer, Hannah, Berger, Benjamin, Zinke, Jan, Alberty, Anke, Kleiter, Ingo, Schneider-Gold, Christiane, Roth, Christian, Dunkel, Juliane, Steinbrecher, Andreas, Thieme, Andrea, Schlachetzki, Felix, Linker, Ralf A., Angstwurm, Klemens, Meisel, Andreas und Neumann, Bernhard
(2021)
MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation.
Journal of Neurology 268, S. 4824-4833.
Veröffentlichungsdatum dieses Volltextes: 14 Mai 2021 05:47
Artikel
DOI zum Zitieren dieses Dokuments: 10.5283/epub.45681
Zusammenfassung
Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in similar to 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a ...
Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in similar to 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a retrospective German multicenter study. We identified 19 MuSK-AB associated MCs in 15 patients and 161 MCs in 144 patients with AchR-ABs only. In contrast to patients with AchR-AB, MuSK-AB patients were more often female (p = 0.05, OR = 2.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (p = 0.04, OR = 3.25). MuSK-AB patients suffer more often from multiple chronic disease (p = 0.016, OR = 4.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0 +/- 53.1 vs. 17.4 +/- 18; p < 0.0001), days on an intensive care unit (ICU) (45.3 +/- 49.5 vs. 21.2 +/- 19.7; p < 0.0001), and hospital-length of stay (LOS) (55.9 +/- 47.6 vs. 28.8 +/- 20.9 days; p < 0.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients' outcome with both antibodies was similar to AchR-MCs. Furthermore, our data showed a shortened duration of MV after treatment with plasma exchanging therapies compared to treatment with intravenous immunoglobulin in MuSK-MCs. We conclude that MuSK-AB-status is associated with a longer need of MV, ICU-LOS, and hospital-LOS in MC, and therefore recommend early initiation of a disease-specific therapy.
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| Dokumentenart | Artikel | ||||
| Titel eines Journals oder einer Zeitschrift | Journal of Neurology | ||||
| Verlag: | SPRINGER HEIDELBERG | ||||
|---|---|---|---|---|---|
| Ort der Veröffentlichung: | HEIDELBERG | ||||
| Band: | 268 | ||||
| Seitenbereich: | S. 4824-4833 | ||||
| Datum | 10 Mai 2021 | ||||
| Institutionen | Medizin > Lehrstuhl für Neurologie | ||||
| Identifikationsnummer |
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| Stichwörter / Keywords | GRAVIS; AUTOANTIBODIES; MORTALITY; Myasthenia gravis; Myasthenic crisis; Autoimmune diseases; Antibody status; MuSK-antibodies; Outcome | ||||
| Dewey-Dezimal-Klassifikation | 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin | ||||
| Status | Veröffentlicht | ||||
| Begutachtet | Ja, diese Version wurde begutachtet | ||||
| An der Universität Regensburg entstanden | Zum Teil | ||||
| URN der UB Regensburg | urn:nbn:de:bvb:355-epub-456817 | ||||
| Dokumenten-ID | 45681 |
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